Background : Epithelioid hemangioendothelioma (HEHE) is a rare tumor of vascular endothelial cells that may involve the liver, lung, spleen, heart, and bone. The treatment options used in case of liver involvement include liver transplantation (LT), liver resection, local treatments, chemotherapy, or simple observation without treatment.

Methods : This retrospective study included 5 patients with the diagnosis of Hepatic Epithelioid Hemangioendothelioma (HEHE) based on preoperative biopsy and post-operative histopathology, treated between 1999 to 2023 at IRCCS-ISMETT in Palermo, Italy. All patients were treated with liver transplantation (LT) and two of these with Sirolimus before and after LT because of metastatic disease, with lymph nodes and lung involvement.

Results : All the patients were monitored by 3-monthly surveillance imaging, in the first year after LT, with radiologic stability and no recurrence of the disease until the last follow-up.

Conclusions : Although based on a small group of patients, our results suggest an important role of combined treatment with Sirolimus and liver transplantation for metastatic HEHE. According to our preliminary results, we suggest an initial observation period of 1-3 months in order to assess tumor biology, in case of tumor progression (an increase of the lesions in 3 months), patients should receive neoadjuvant therapy and subsequent LT. The role of combination treatment for metastatic HEHE requires further studies with a longer follow-up that should provide more data about the patient population.